Background:: Diseases and tumors of the appendix vermiformis are very rare, except acute appendicitis. Objectives:: This retrospective study was conducted to document the unusual findings in appendectomy specimens. Patients and Methods:: Data of 1466 adult patients were gathered retrospectively. Appendectomy was performed in 1169 and in 297 patients following a diagnosis of acute appendicitis and during other abdominal operations, respectively. The data of 57 (3.88 %) patients who were pathologically reported to have unusual appendix findings were retrospectively collected. The records were analyzed according to patients’ age, gender, clinical presentations, operative reports, pathological reports and follow up. Results:: Unusual pathologic examination findings were detected in the appendectomy specimens of 57 patients with a mean age of 48.34 ± 19. Twenty-nine patients (50.8 %) were male and 28 (49.2 %) were female. Normal appendix tissues were observed in specimens of 26 (45.6 %) patients and inflamed appendix in 31 (54.3 %). The most common unusual finding was parasitic diseases of the intestine. Pathological diagnosis of malignancy and benign features were reported in specimens of 14 and 43 patients, respectively. Macroscopic evaluation of appendectomy specimens during surgery might result in negligence of the presence of unusual pathology. Conclusions:: Even if the macroscopic appearance of the specimen is normal or acute appendicitis, we suggest routine histopathological examination.